microtia and meatal stenosis or atresia with malformation of the middle
and/or inner ears.
iv. Van der Hoeve^s syndrome in which there is osteogenesis imperfecta,
blue sclerotics and conductive deafness.
Fig. 213. Congenital deaf-mutism due to faulty
development of membranous cochlea. Hori-
zontal section through upper part of left basal
coil. 1, Dilated cochlear duct; 2, Split in bony
capsule (artefact); 3, Adhesion between
membrana tectoria and stria vascularis; 4,
Rudimentary Corti's organ.
Fig. 215. Same case as Figs. 213, 214, 216.
Vertical section through upper part of right
basal coil. 1, Reissner's membrane depressed
and attached to malformed Corti's organ; 2,
Position of stria vascularis, which is absent;
3, Position of spiral prominence; 4, Basilar
membrane; 5, Membrana tectoria tucked into
spiral sulcus. It will be seen that there is a
complete malformation of the epithelium
lining the cochlear canal.
Fig. 214. Same case as Figs. 213, 215, 216.
Horizontal section through lower part of left
middle coil. 1, Dilated cochlear duct; 2.
Membrana tectoria lying on the apex of the
limb us; 3, Rudimentary Corti's organ. The
spiral ligament shows oedematous degeneration.
The stria vascularia appears to be well formed.
Fig. 216. Same case as Figs. 213, 214, 215.
Vertical section through lower part of right
middle coil. 1, Membrana tectoria, which lies
between the origin of Reissner's membrane and
the limbus; 2, Rudimentary Corti's organ; 3,
Great proliferation of stria vascularis, which
occupies the outer third of the narrow cochlear
v. Dyostosis cleidocranialis which consists of absence of the clavicles and
abnormalities of the skull, spine and pelvis. There may be stenosis of the
bony meatus with hearing loss.
vi. Hunter's syndrome is a recessive type of gargoylism associated with